POEMS syndrome, which is also known as Crow–Fukase syndrome, Takatsuki disease, or PEP syndrome, is a rare blood disorder that damages the patient’s nerves and affects many other parts of the body. It is defined as the combination of a plasma-cell proliferative disorder, polyneuropathy, and effects on many other organ systems.
POEMS syndrome can be misdiagnosed because the signs and symptoms mimic those of other disorders. POEMS syndrome progresses rapidly and may become life-threatening, so early diagnosis is important.
The term "POEMS" is an acronym that derives from for the following signs and symptoms:
- Polyneuropathy. It is a defining feature and it is usually symmetrical and distal. There is a combination of demyelination and axonal damage, and sensory, motor and autonomic neurones are all affected. The typical symptoms are therefore of numbness, tingling and weakness in the feet, later affecting the legs and hands. Pain is unusual, but the weakness may eventually become severe and disabling. The autonomic neuropathy may cause excessive sweating and erectile dysfunction.
- Organomegaly. It is characterized by an enlarged spleen, liver or lymph nodes.
- Endocrinopathy. Abnormal hormone levels that can result in underactive thyroid gland, diabetes, sexual problems, fatigue, swelling in your limbs and problems with metabolism and other essential functions.
- Monoclonal plasma-proliferative disorder. It is the second defining feature and most common plasma-cell proliferative disorder associated with POEMS syndrome, present in more than half of all cases. The characteristic of those cases are the abnormal bone marrow cells that produce a monoclonal protein, which can be found in the bloodstream.
- Skin changes. A very wide variety of skin problems have been reported in association with POEMS syndrome. The most common is non-specific hyperpigmentation, when the fingernails may be clubbed or white. In addition, the patient may have more color than normal on the skin; there may be thickening of the skin; excess hair or hair in unusual places; skin angiomas or haemangiomas or there may be changes reminiscent of scleroderma.
Other symptoms include papilloedema, pulmonary diseases that occuer at more severe stages of the illness and affect the lungs; oedema (which is a leakage of fluid into the tissues) and hormonal problems such as: endocrinopathy, type 2 diabetes, hypothyroidism and adrenal insufficiency;
Usually POEMS syndrome begins in middle ages. The average age at onset is 50. Apart from that, PEOMS syndrome affects up to twice as many men as women.
If POEMS syndrome is left untreated, it will most probably progress and often be fatal for the patient, with only 60% of sufferers remaining alive five years after onset. However, the symptoms can improve if the blood disorder is successfully treated.
Uncommon features or complications are:
- Respiratory – pulmonary hypertension, restrictive lung disease.
- Thrombosis – arterial or venous.
- Renal impairment.
- Congestive cardiac failure.
- A capillary leak-like syndrome